Cellular Defects in CVID Patients with Chronic Lung Disease in the USIDNET Registry

The USIDNET Consortium

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Purpose: Chronic lung disease is the most common cause of morbidity and mortality in patients with common variable immunodeficiency (CVID). While biomarkers exist to predict non-infectious complications, the unique features that define CVID patients with chronic lung disease are not well understood. Methods: We analyzed data from CVID patients from the retrospective USIDNET (United States Immunodeficiency Network) patient database. Patients were categorized into 3 phenotypes for comparison: (1) CVID without chronic lung disease, (2) CVID with bronchiectasis only, and (3) CVID with interstitial lung disease (ILD) with or without bronchiectasis. Among these groups, differences were assessed in demographics, comorbidities, infections, treatments, and peripheral blood immune measures. We analyzed 1518 CVID patients which included 1233 (81.2%) without chronic lung disease, 147 (9.7%) with bronchiectasis only, and 138 (9.1%) with interstitial lung disease. Results: Patients with ILD had lower CD3+ cell counts (P =.001), CD4+ cell counts (P <.05), and CD8+ cell counts (P <.001) compared with patients without lung disease. Additionally, there was significantly more CVID patients with ILD with pneumonia (P <.001), herpes viruses (P =.01) and fungal infections (P <.001) compared with patients with CVID without chronic lung disease. Conclusion: This analysis suggests that patients with chronic lung disease may be more likely to have lower peripheral T cell counts and complications of those defects compared with CVID patients without chronic lung disease.

Original languageEnglish (US)
JournalJournal of Clinical Immunology
DOIs
StatePublished - Jan 1 2019

Fingerprint

Common Variable Immunodeficiency
Lung Diseases
Registries
Chronic Disease
Interstitial Lung Diseases
Bronchiectasis
Cell Count
Mycoses
CD4 Lymphocyte Count
Comorbidity
Pneumonia

Keywords

  • CVID
  • Common variable immunodeficiency
  • GLILD
  • USIDNET
  • autoimmunity
  • granulomatous lymphocytic interstitial lung disease

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

@article{9236c9e0fff243fcb5bbff1a6f7c466e,
title = "Cellular Defects in CVID Patients with Chronic Lung Disease in the USIDNET Registry",
abstract = "Purpose: Chronic lung disease is the most common cause of morbidity and mortality in patients with common variable immunodeficiency (CVID). While biomarkers exist to predict non-infectious complications, the unique features that define CVID patients with chronic lung disease are not well understood. Methods: We analyzed data from CVID patients from the retrospective USIDNET (United States Immunodeficiency Network) patient database. Patients were categorized into 3 phenotypes for comparison: (1) CVID without chronic lung disease, (2) CVID with bronchiectasis only, and (3) CVID with interstitial lung disease (ILD) with or without bronchiectasis. Among these groups, differences were assessed in demographics, comorbidities, infections, treatments, and peripheral blood immune measures. We analyzed 1518 CVID patients which included 1233 (81.2{\%}) without chronic lung disease, 147 (9.7{\%}) with bronchiectasis only, and 138 (9.1{\%}) with interstitial lung disease. Results: Patients with ILD had lower CD3+ cell counts (P =.001), CD4+ cell counts (P <.05), and CD8+ cell counts (P <.001) compared with patients without lung disease. Additionally, there was significantly more CVID patients with ILD with pneumonia (P <.001), herpes viruses (P =.01) and fungal infections (P <.001) compared with patients with CVID without chronic lung disease. Conclusion: This analysis suggests that patients with chronic lung disease may be more likely to have lower peripheral T cell counts and complications of those defects compared with CVID patients without chronic lung disease.",
keywords = "CVID, Common variable immunodeficiency, GLILD, USIDNET, autoimmunity, granulomatous lymphocytic interstitial lung disease",
author = "{The USIDNET Consortium} and Kellner, {Erinn S.} and Ramsay Fuleihan and Charlotte Cunningham-Rundles and Wechsler, {Joshua B.}",
year = "2019",
month = "1",
day = "1",
doi = "10.1007/s10875-019-00657-w",
language = "English (US)",
journal = "Journal of Clinical Immunology",
issn = "0271-9142",
publisher = "Springer New York",

}

Cellular Defects in CVID Patients with Chronic Lung Disease in the USIDNET Registry. / The USIDNET Consortium.

In: Journal of Clinical Immunology, 01.01.2019.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Cellular Defects in CVID Patients with Chronic Lung Disease in the USIDNET Registry

AU - The USIDNET Consortium

AU - Kellner, Erinn S.

AU - Fuleihan, Ramsay

AU - Cunningham-Rundles, Charlotte

AU - Wechsler, Joshua B.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Purpose: Chronic lung disease is the most common cause of morbidity and mortality in patients with common variable immunodeficiency (CVID). While biomarkers exist to predict non-infectious complications, the unique features that define CVID patients with chronic lung disease are not well understood. Methods: We analyzed data from CVID patients from the retrospective USIDNET (United States Immunodeficiency Network) patient database. Patients were categorized into 3 phenotypes for comparison: (1) CVID without chronic lung disease, (2) CVID with bronchiectasis only, and (3) CVID with interstitial lung disease (ILD) with or without bronchiectasis. Among these groups, differences were assessed in demographics, comorbidities, infections, treatments, and peripheral blood immune measures. We analyzed 1518 CVID patients which included 1233 (81.2%) without chronic lung disease, 147 (9.7%) with bronchiectasis only, and 138 (9.1%) with interstitial lung disease. Results: Patients with ILD had lower CD3+ cell counts (P =.001), CD4+ cell counts (P <.05), and CD8+ cell counts (P <.001) compared with patients without lung disease. Additionally, there was significantly more CVID patients with ILD with pneumonia (P <.001), herpes viruses (P =.01) and fungal infections (P <.001) compared with patients with CVID without chronic lung disease. Conclusion: This analysis suggests that patients with chronic lung disease may be more likely to have lower peripheral T cell counts and complications of those defects compared with CVID patients without chronic lung disease.

AB - Purpose: Chronic lung disease is the most common cause of morbidity and mortality in patients with common variable immunodeficiency (CVID). While biomarkers exist to predict non-infectious complications, the unique features that define CVID patients with chronic lung disease are not well understood. Methods: We analyzed data from CVID patients from the retrospective USIDNET (United States Immunodeficiency Network) patient database. Patients were categorized into 3 phenotypes for comparison: (1) CVID without chronic lung disease, (2) CVID with bronchiectasis only, and (3) CVID with interstitial lung disease (ILD) with or without bronchiectasis. Among these groups, differences were assessed in demographics, comorbidities, infections, treatments, and peripheral blood immune measures. We analyzed 1518 CVID patients which included 1233 (81.2%) without chronic lung disease, 147 (9.7%) with bronchiectasis only, and 138 (9.1%) with interstitial lung disease. Results: Patients with ILD had lower CD3+ cell counts (P =.001), CD4+ cell counts (P <.05), and CD8+ cell counts (P <.001) compared with patients without lung disease. Additionally, there was significantly more CVID patients with ILD with pneumonia (P <.001), herpes viruses (P =.01) and fungal infections (P <.001) compared with patients with CVID without chronic lung disease. Conclusion: This analysis suggests that patients with chronic lung disease may be more likely to have lower peripheral T cell counts and complications of those defects compared with CVID patients without chronic lung disease.

KW - CVID

KW - Common variable immunodeficiency

KW - GLILD

KW - USIDNET

KW - autoimmunity

KW - granulomatous lymphocytic interstitial lung disease

UR - http://www.scopus.com/inward/record.url?scp=85068207199&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85068207199&partnerID=8YFLogxK

U2 - 10.1007/s10875-019-00657-w

DO - 10.1007/s10875-019-00657-w

M3 - Article

C2 - 31250334

AN - SCOPUS:85068207199

JO - Journal of Clinical Immunology

JF - Journal of Clinical Immunology

SN - 0271-9142

ER -